Moore LR,Rajpal G,Dillingham IT,et al.Evaluation of Antisense Oligonucleotides Targeting ATXN3 in SCA3 Mouse Models[J].Mol Ther Nucleic Acids,2017,7:200-210.
[2]
Kuiper EF,de Mattos EP,Jardim LB,et al.Chaperones in Polyglutamine Aggregation:Beyond the QStretch[J].Front Neurosci,2017,11:145.
[3]
Ramani B,Harris GM,Huang R,et al.A knockin mouse model of spinocerebellar ataxia type 3exhibits prominent aggregate pathology and aberrant splicing of the disease gene transcript[J].Hum Mol Genet,2017,26(16):3 232-3 233.
[4]
Andreia TC,Ana J,Sofia E,et al.Serotonergic signalling suppresses ataxin 3aggregation and neurotoxicity in animal models of Machado-Joseph disease[J].Brain,2015,138(Pt11):3 221-3 237.
Chou AH,Chen YL,Chiu CC,et al.T1-11 and JMF1907ameliorate polyglutamine-expanded ataxin-3-induced neurodegeneration,transcriptional dysregulation and ataxic symptom in the SCA3transgenic mouse[J].Neuropharmacology,2015,99:308-317.
[7]
Silva-Fernandes A,Costa MdC,Duarte-Silva S,et al.Motor uncoordination and neuropathology in a transgenic mouse model of Machado-Joseph disease lacking intranuclear inclusions and ataxin-3cleavage products[J].Neurobiol Dis,2010,40(1):163-176.
Boy J,Schmidt T,Wolburg H,et al.Reversibility of symptoms in a conditional mouse model of spinocerebellar ataxia type3[J].Hum Mol Genet,2009,18(22):4 282-4 295.
[10]
Boy J,Schmidt T,Schumann U,et al.A transgenic mouse model of spinocerebellar ataxia type3resembling late disease onset and gender-specific instability of CAG repeats[J].Neurobiol Dis,2010,37(2):284-293.